Low rate of cardiac events in first-degree relatives of diagnosis-negative young sudden unexplained death syndrome victims during follow-up

被引:28
作者
van der Werf, Christian [1 ]
Stiekema, Lotte [1 ]
Tan, Hanno L. [1 ]
Hofman, Nynke [2 ]
Alders, Marielle [2 ]
van der Wal, Allard C. [3 ]
van Langen, Irene M. [4 ]
Wilde, Arthur A. M. [1 ,5 ]
机构
[1] Univ Amsterdam, Acad Med Ctr, Dept Cardiol, Ctr Heart, NL-1105 AZ Amsterdam, Netherlands
[2] Univ Amsterdam, Acad Med Ctr, Dept Clin Genet, NL-1105 AZ Amsterdam, Netherlands
[3] Univ Amsterdam, Acad Med Ctr, Dept Pathol, NL-1105 AZ Amsterdam, Netherlands
[4] Univ Groningen, Univ Med Ctr Groningen, Dept Genet, Groningen, Netherlands
[5] King Abdulaziz Univ, Princess Al Jawhara Albrahim Ctr Excellence Res H, Jeddah 21413, Saudi Arabia
关键词
Sudden unexplained death syndrome; Sudden cardiac death; Arrhythmia; Genetics; CARDIOVASCULAR-DISEASE; ARREST; ADULTS; RISK;
D O I
10.1016/j.hrthm.2014.05.028
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
BACKGROUND Sudden unexplained death syndrome (SUDS) in young individuals often results from inherited cardiac disease. Accordingly, comprehensive examination in surviving first-degree relatives unmasks such disease in approximately 35% of the families. It is unknown whether individuals from diagnosisnegative families are at risk of developing manifest disease or cardiac events during follow-up. OBJECTIVE This study aimed to study the prognosis of first-degree relatives of young SUDS victims, in whom the initial cardiologic and genetic examination did not lead to a diagnosis. METHODS We retrieved vital status of surviving first-degree relatives from 83 diagnosis-negative families who presented to our cardiogenetics department between 1996 and 2009 because of SUDS in > 1 relatives aged 1-50 years. Moreover, we contacted relatives who previously visited our center for detailed information. RESULTS We obtained detailed information (median follow-up 6.6 years; interquartile range 4.7-9.6 years) in 340 of 417 first-degree relatives (81.5%) from 77 of 83 families (92.8%). Vital status, available in 405 relatives (97.1%), showed that 20 relatives (4.9%) died during follow-up, including 1 natural death before the age of 50. This girl belonged to a family with multiple cases of idiopathic ventricular fibrillation and SUDS, including another successfully resuscitated sibling during follow-up. Two hundred thirty-four of 340 first-degree relatives (68.8%) underwent cardiologic examination. Of these, 76 (32.5 /0) were reevaluated. Inherited cardiac disease was diagnosed in 3 families (3.6%). CONCLUSION In first-degree relatives of young SUDS victims with no manifest abnormalities during the initial examination, the risk of developing manifest inherited cardiac disease or cardiac events during follow-up is low. This does not apply to families with obvious familial SUDS.
引用
收藏
页码:1728 / 1732
页数:5
相关论文
共 21 条
[1]   Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome [J].
Behr, E ;
Wood, DA ;
Wright, M ;
Syrris, P ;
Sheppard, MN ;
Casey, A ;
Davies, MJ ;
McKenna, W .
LANCET, 2003, 362 (9394) :1457-1459
[2]   Sudden arrhythmic death syndrome: familial evaluation identifies inheritable heart disease in the majority of families [J].
Behr, Elijah R. ;
Dalageorgou, Chrysouta ;
Christiansen, Michael ;
Syrris, Petros ;
Hughes, Sian ;
Esteban, Maria T. Tome ;
Rowland, Edward ;
Jeffery, Steve ;
McKenna, William J. .
EUROPEAN HEART JOURNAL, 2008, 29 (13) :1670-1680
[3]   The clinical management of relatives of young sudden unexplained death victims; implantable defibrillators are rarely indicated [J].
Caldwell, Jane ;
Moreton, Natalie ;
Khan, Naz ;
Kerzin-Storrar, Lauren ;
Metcalfe, Kay ;
Newman, William ;
Garratt, Clifford J. .
HEART, 2012, 98 (08) :631-636
[4]   Does sports activity enhance the risk of sudden death in adolescents and young adults? [J].
Corrado, D ;
Basso, C ;
Rizzoli, G ;
Schiavon, M ;
Thiene, G .
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2003, 42 (11) :1959-1963
[5]   Causes of sudden cardiac death in young Australians [J].
Doolan, A ;
Langlois, N ;
Semsarian, C .
MEDICAL JOURNAL OF AUSTRALIA, 2004, 180 (03) :110-112
[6]   SUDDEN UNEXPECTED DEATH IN PERSONS LESS-THAN-40 YEARS OF AGE [J].
DRORY, Y ;
TURETZ, Y ;
HISS, Y ;
LEV, B ;
FISMAN, EZ ;
PINES, A ;
KRAMER, MR .
AMERICAN JOURNAL OF CARDIOLOGY, 1991, 68 (13) :1388-1392
[7]   Sudden death in young adults: A 25-year review of autopsies in military recruits [J].
Eckart, RE ;
Scoville, SL ;
Campbell, CL ;
Shry, EA ;
Stajduhar, KC ;
Potter, RN ;
Pearse, LA ;
Virmani, R .
ANNALS OF INTERNAL MEDICINE, 2004, 141 (11) :829-834
[8]   Strategy for clinical evaluation and screening of sudden cardiac death relatives [J].
Ferrero-Miliani, Laura ;
Holst, Anders Gaarsdal ;
Pehrson, Steen ;
Morling, Niels ;
Bundgaard, Henning .
FUNDAMENTAL & CLINICAL PHARMACOLOGY, 2010, 24 (05) :619-635
[9]   Active Cascade Screening in Primary Inherited Arrhythmia Syndromes [J].
Hofman, Nynke ;
Tan, Hanno L. ;
Alders, Marielle ;
van Langen, Irene M. ;
Wilde, Arthur A. M. .
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 2010, 55 (23) :2570-2576
[10]   Sudden cardiac death in the young: a clinical genetic approach [J].
Ingles, J. ;
Semsarian, C. .
INTERNAL MEDICINE JOURNAL, 2007, 37 (01) :32-37